By Roland T. Jung
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Additional resources for Endocrine Problems in Cancer. Molecular Basis and Clinical Management
Adv. Cancer Res; 33: 39-75. R. (1980). A C T H secreting medullary carcinoma of the thyroid: Monitoring of clinical course with calcitonin and cortisol assays and immunohistochemical studies. Cancer; 46: 2667-70. , Bentall M. 5 Peckham M J . (1976). Lancet; 433-6. , Landon J. (1976). Chemical characteristics of ectopie A C T H purified from a malignant thymic carcinoid tumour. J. Clin. EndocrinoL Metab; 43: 831-5. W. (1982). Laboratory diagnosis of gastrinoma. II A prospective study of gastrin challenge tests.
This also is unexplained. GONADAL DYSGENESIS For a full account of neoplasia in disorders of abnormal sexual differen tiation, the reader is urged to consult Simpson and Photopoulos (1976). What follows is a highly selective account of a complex subject. In individuals with gonadal dysgenesis, the gonads are represented by streaks of connective tissue lacking germ cells. , 1975). Two forms are distinguished: (1) cytogenetic, associated with monosomy for the X chromosome (45,X) or structural rearrangements of the X or Y chromosomes ; and (2) genetic, in which the gonadal dysgenesis is associated with an inherited mutant gene, but there is a normal female (46,XX) or male (46,XY) chromosome component.
Although the classical description of MEN II is of families with both M T C and phaeochromocytoma, perhaps as many as 50% of families with M T C have no evidence of phaeochromocytoma, even after Genetic Problems of Endocrine Significance 43 careful screening; familial M T C should therefore be recognised as a separate entity. It is possible also that the description of MEN II as autosomal dominant with high penetrance is an over-simplification. Data from Norway (Normann, 1977) and our own experience (unpublished) indicates that penetrance may sometimes be incomplete, in that family members who were clinically normal aged 65 or more have transmitted the gene; and in some families the phenotypic expression of the syndrome seems to differ in different branches.
Endocrine Problems in Cancer. Molecular Basis and Clinical Management by Roland T. Jung